Long-term outcome of congenital intestinal pseudoobstruction.

We evaluated 85 children with congenital chronic intestinal pseudoobstruction (CIP) over the past 10 years. Twelve (14%) were born prematurely. One had a family history of CIP. Six had systemic diseases. Thirty-five (41%) had urinary bladder involvement. Manometric features were consistent with myopathy in 32, neuropathy in 48, and mixed disease in 5. Of 48 patients with neuropathy, 6 had urinary bladder involvement (12.5%) (P < 0.0001 vs myopathy), and 10 had malrotation (21%) (P = NS vs myopathy). Upon referral, 53 (62%) were dependent on partial or total parenteral nutrition (PN). At the time of chart review (median 25 months after evaluation), 22 patients had died, 14 of whom were on total PN, 13 of them died because of PN-related complications and 1 died of sepsis. Three others died of sepsis while on partial PN (P = 0.007 vs mortality in patients fed enterally) and five died after small bowel transplantation. In conclusion, in children with congenital CIP, the risk for prematurity is increased twofold, the majority of cases are sporadic, abnormal bladder function is more common in myopathic CIP, and complications related to parenteral nutrition are the main cause of death in children with CIP.

Manometric evaluation of gastrointestinal motility in a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS).

We report on a manometric study of the esophagus, stomach, duodenum, jejunum and anorectum in a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). An esophageal examination showed normal esophageal contractions and a positive rectoanal reflex was obtained in an anorectal manometry. However, the continuous fasting manometric recording of the antrum, duodenum and jejunum lasting a total of 12 hours revealed a severe motility disturbance of the gastrointestinal tract. During the examination a band of low amplitude contractions was observed in the antrum only once and it was followed by small rhythmic contractions of the duodenum, but none of propagated or spontaneous contractions were seen in the jejunum.

[Megacystis microcolon intestinal hypoperistalsis syndrome: A neuropathy?]. / Das Megazystis-Mikrokolon-intestinale Hypoperistaltik-Syndrom: Eine Neuropathie?

2 cases of megacystis microcolon intestinal hypoperistalsis are presented. A female newborn was capable of being fed completely enterally after three months. Laparotomy was not performed. A male newborn was subjected to laparotomy after 3 days and an ileal stoma was applied. The infant died after 6 months of complete parenteral feeding without any peristalsis having been initiated. Biopsies of the colon and small intestine of the patient showed normal HE staining findings. Histochemical examination revealed type B neuronal dysplasia with neuronal hypogenesis. The findings of 27 cases described in the literature are discussed with special reference to the histological findings of the intestinal wall.